Abnormalities in pharyngeal arch-derived structures in SATB2-associated syndrome.

SATB2-associated syndrome (SAS, glass syndrome, OMIM#612313) is a neurodevelopmental autosomal dominant disorder with frequent craniofacial abnormalities including palatal and dental anomalies. To assess the role of Satb2 in craniofacial development, we analyzed mutant mice at different stages of development. Here, we show that Satb2 is broadly expressed in early embryonic mouse development including the mesenchyme of the second and third arches. Satb2-/- mutant mice exhibit microglossia, a shortened lower jaw, smaller trigeminal ganglia, and larger thyroids. We correlate these findings with the detailed clinical phenotype of four individuals with SAS and remarkable craniofacial phenotypes with one requiring mandibular distraction in childhood. We conclude that the mouse and patient data presented support less well-described phenotypic aspects of SAS including mandibular morphology and thyroid anatomical/functional issues.

Identification of a de novo PUF60 variant associated with craniofacial microsomia.

Craniofacial microsomia (CFM), also known as the oculo-auriculo-vertebral spectrum, is a congenital disorder characterized by hypoplasia of the mandible and external ear due to tissue malformations originating from the first and second branchial arches. However, distinguishing it from other syndromes of branchial arch abnormalities is difficult, and causal variants remain unidentified in many cases. In this report, we performed an exome sequencing analysis of a Brazilian family with CFM. The proband was a 12-month-old boy with clinical findings consistent with the diagnostic criteria for CFM, including unilateral mandibular hypoplasia, microtia, and external auditory canal abnormalities. A heterozygous de novo nonsense variant (c.713C>G, p.S238*) in PUF60 was identified, which was predicted to be pathogenic in silico. PUF60 has been reported as a causal gene in Verheij syndrome, but not in CFM. Although the boy showed craniofacial abnormalities and developmental delay that overlapped with Verheij syndrome, the facial asymmetry with unilateral hypoplasia of the mandible observed in this case did not match the previously reported phenotypes of PUF60 variants. Our findings expand the phenotypic range of PUF60 variants that cover CFM and Verheij syndrome.

Machine Learning Effectively Diagnoses Mandibular Deformity Using Three-Dimensional Landmarks.

BACKGROUND: Jaw deformity diagnosis requires objective tests. Current methods, like cephalometry, have limitations. However, recent studies have shown that machine learning can diagnose jaw deformities in two dimensions. Therefore, we hypothesized that a multilayer perceptron (MLP) could accurately diagnose jaw deformities in three dimensions (3D). PURPOSE: Examine the hypothesis by focusing on anomalous mandibular position. We aimed to: (1) create a machine learning model to diagnose mandibular retrognathism and prognathism; and (2) compare its performance with traditional cephalometric methods. STUDY DESIGN, SETTING, SAMPLE: An in-silico experiment on deidentified retrospective data. The study was conducted at the Houston Methodist Research Institute and Rensselaer Polytechnic Institute. Included were patient records with jaw deformities and preoperative 3D facial models. Patients with significant jaw asymmetry were excluded. PREDICTOR VARIABLES: The tests used to diagnose mandibular anteroposterior position are: (1) SNB angle; (2) facial angle; (3) mandibular unit length (MdUL); and (4) MLP model. MAIN OUTCOME VARIABLE: The resultant diagnoses: normal, prognathic, or retrognathic. COVARIATES: None. ANALYSES: A senior surgeon labeled the patients' mandibles as prognathic, normal, or retrognathic, creating a gold standard. Scientists at Rensselaer Polytechnic Institute developed an MLP model to diagnose mandibular prognathism and retrognathism using the 3D coordinates of 50 landmarks. The performance of the MLP model was compared with three traditional cephalometric measurements: (1) SNB, (2) facial angle, and (3) MdUL. The primary metric used to assess the performance was diagnostic accuracy. McNemar's exact test tested the difference between traditional cephalometric measurement and MLP. Cohen's Kappa measured inter-rater agreement between each method and the gold standard. RESULTS: The sample included 101 patients. The diagnostic accuracy of SNB, facial angle, MdUL, and MLP were 74.3, 74.3, 75.3, and 85.2%, respectively. McNemar's test shows that our MLP performs significantly better than the SNB (P = .027), facial angle (P = .019), and MdUL (P = .031). The agreement between the traditional cephalometric measurements and the surgeon's diagnosis was fair. In contrast, the agreement between the MLP and the surgeon was moderate. CONCLUSION AND RELEVANCE: The performance of the MLP is significantly better than that of the traditional cephalometric measurements.

Severity of Mandibular Dysmorphology in Treacher Collins Syndrome for Stratification of Perioperative Airway Risk.

BACKGROUND: Patients with Treacher Collins syndrome (TCS) and attendant airway dysmorphology may be predisposed to airway complications in the perioperative period. However, limited data correlates severity of mandibular hypoplasia and airway status. This study aims to improve risk stratification for perioperative airway insufficiency in TCS by using a previously proposed mandibular severity index. METHODS: Patient demographics, perioperative airway status, difficulty of intubation, and Cormack Lehane grade were collected and compared using a TCS mandibular hypoplasia severity grading scale in patients with TCS treated between 2000 and 2022. RESULTS: Twenty-six patients underwent 222 procedures with institutional mandibular severity gradings as follows: 23% Grade I, 31% Grade II, 39% Grade III, 8% Grade IV. Our severity index was associated with intubation difficulty ( P <0.001) and difficult airway status ( P <0.001), with 72% of difficult airways found in grade III and grade IV patients. Mandibular retrusion and ramal hypoplasia subscores were positively correlated with measures of airway severity ( P <0.001), whereas the gonial angle was negatively correlated ( P <0.001). Age was negatively correlated with difficult visualization for endotracheal intubation ( P =0.02) but had no association with difficult airway status ( P =0.2). CONCLUSIONS: This study found a positive correlation between severity of maxillomandibular dysmorphology and perioperative airway difficulty in TCS patients. Our findings suggest that severely affected patients require heightened vigilance throughout life, as difficult airways may not completely resolve with aging. Given the risk of morbidity and mortality associated with airway complications, proper identification and preparation for challenging airways is critical for TCS patients.

Jaw Thrust: A Simple Predictor of Success in Mandibular Distraction Osteogenesis.

BACKGROUND: The hypoplastic mandible in the congenital condition Pierre Robin sequence (PRS) displaces the base of the tongue posteriorly, which results in upper airway obstruction (UAO) that can potentially be corrected with mandibular distraction osteogenesis (MDO). Jaw thrust (JT) is routinely performed during evaluation of the airway; similar to MDO, it projects the mandible and tongue anteriorly to open the airway. The authors demonstrate that JT can be used as a criterion to predict successful MDO outcomes in infants with PRS. METHODS: The study was a single-center, retrospective chart review of infants diagnosed with PRS between 2016 and 2023. Data regarding their demographics, comorbid diagnoses, JT success, airway anomalies, laryngeal grade of view, apnea-hypopnea index, and perioperative course were statistically analyzed. RESULTS: Of the 16 patients included in the study, 11 had successful relief of their airway obstruction with JT and proceeded with MDO. The unsuccessful JT group had significantly greater proportions of females, birth prematurity, gastrostomies, tracheostomies, and longer hospital stays. In the successful JT group, both the mean laryngeal grade of view ( P =0.029) and mean apnea-hypopnea index ( P =0.025) improved significantly post-MDO. Post-MDO tracheostomy was also avoided in all but 1 patient who was not previously tracheostomized. CONCLUSIONS: There is no widely accepted algorithm to guide craniofacial surgeons on the optimal intervention for relieving UAO in infants with PRS. In our institutional experience, patients whose preoperative JT relieved UAO also successfully relieved UAO with MDO. In patients with PRS, JT may be a useful criterion for selecting appropriate candidates for MDO.

Three-dimensional evaluation of dental characteristics in patients with Cleidocranial dysplasia.

BACKGROUND: Cleidocranial dysplasia (CCD) is an autosomal dominant hereditary disorder. Besides skeletal abnormalities, CCD is often associated with dental complications, such as multiple supernumerary teeth and permanent teeth impaction or delayed eruption. METHODS: Supernumerary teeth of axial, sagittal and coronal CBCT view was characterized in detail and 3D image reconstruction was performed. Number and location of teeth, morphology of supernumerary teeth, positional relationship between supernumerary and adjacent permanent teeth, direction of supernumerary teeth in CCD patients were analyzed. RESULTS: The mean age of the 3 CCD patients in this study was 16.7 years. Among 36 supernumerary teeth, the majority of them were identified as apical side located and lingual side located. Normal orientation was the most common type in this study, followed by sagittal orientation, and horizontal orientation. Horizontal orientation teeth were all distributed in the mandible. Supernumerary teeth exhibited significantly shorter crown and dental-root lengths, as well as smaller crown mesiodistal and buccolingual diameters (P < 0.01). There was no difference in the number of supernumerary teeth between the maxilla and mandible, and the premolars region had the largest number of supernumerary teeth and the incisor region had the smallest number. CONCLUSIONS: This study compares number and location of teeth, morphology of supernumerary teeth, positional relationship between supernumerary and adjacent permanent teeth and direction of supernumerary teeth, this study also provides a reference for the comprehensive evaluation of CCD patients before surgery.

Combined Periodontal-Orthodontic Treatment with Periodontal Corticotomy Regenerative Surgery in an Adult Patient Suffering from Periodontitis and Skeletal Class II Malocclusion: A Case Report with 5-Year Longitudinal Observation.

A thick periodontal phenotype with thick gingiva and alveolar bone volume is required for safe orthodontic tooth movement and long-term stability. A high incidence of dehiscence and fenestration in the labial aspect of mandibular anterior teeth may limit the correction of deformity and orthodontic treatment, especially when the lower anterior teeth are needed to have a large range of movement. This study reports a combination of periodontal therapy and orthodontic therapy with periodontal corticotomy regenerative surgery (PCRS) in a 25-year-old patient suffering from skeletal Class II malocclusion and periodontitis. The patient received periodontal therapy 5 years ago and commenced orthodontic treatment 4.5 years ago. During the 4 years of follow-up for PCRS, the clinical and radiographic evaluations revealed significant improvements in the periodontal phenotype of the mandibular anterior region. The periodontal phenotypes in the mandibular incisors region were all modified from thin to thick. Supplementing orthodontic treatment with labial PCRS could be a promising treatment strategy to maintain long-term periodontal health in adult patients with alveolar deficiency and thin gingiva tissue.

Three-Dimensional Modeling and Quantitative Assessment of Mandibular Volume in Ectodermal Dysplasia: A Case Series.

Background and Objectives: Ectodermal dysplasia (ED)-a genetic disorder-is characterized by severe tooth deficiency. We compared the mandibular volume and the sagittal and horizontal mandibular widths between patients with ED (ED group) and individuals without tooth deficiency (control group) using three-dimensional modeling. We hypothesized that the mandibular volume differs in ED cases owing to congenital tooth deficiency. Materials and Methods: We used previously obtained cone-beam computed tomography (CBCT) images of 13 patients with ED. The control group data comprised retrospective CBCT images of patients of similar age and sex with a skeletal relationship of class 1. Further, using the three-dimensional image analysis software, the tooth crowns were separated from the mandible, the mandible was reconstructed and the gonion-to-gonion distance in the mandible was marked, the distance to the menton point was measured, and the distance between the two condyles was measured and compared with the control group. Results: Overall, 46.2% and 53.8% of the participants were men and women, respectively. In the ED group, the mean age of the participants was 15.46 (range, 6-24) years, and the mean number of mandibular teeth was 4.62. Notably, the edentulous mandible volume of the ED group (27.020 mm3) was statistically significantly smaller than that of the control group (49.213 mm3) (p < 0.001). There was no difference between the two groups in terms of the marked points. For data analysis, the Shapiro-Wilk test, independent samples t-test, and Mann-Whitney U test were used. Conclusions: It has been considered that mandible volume does not develop in ED cases because of missing teeth. Modern practices, such as the CBCT technique and three-dimensional software, may be effective in identifying the true morphologic features, especially in patients with genetic syndromes affecting the maxillofacial structure.

[Growth disturbances of the temporomandibular joint: causes, diagnosis and treatment]. / Groeistoornissen van het kaakgewricht: oorzaken, diagnose en behandeling.

Growth disturbances of the temporomandibular Joint are characterized by mandibular asymmetry, sometimes with secondary maxillar disturbances. Although the clinical symptoms are sometimes quite severe, patients usually have no pain. There are several growth disturbances, but in this article we discuss three particular causes of facial asymmetry, namely hemimandibular growth defects; overdevelopment, underdevelopment and neoplasms of the mandibular joint. Hemimandibular overdevelopment (hyperplasia) is a growth disorder characterized by progressive asymmetry of the mandibula. Hemimandibular hypoplasia, on the other hand, is a growth disorder involving underdevelopment of the condyle mandibulae due to impingement of the growth center and ankylosing. A pronounced asymmetrical face can cause aesthetic problems and always requires diagnostics, because in addition to the hyperplasia and hypoplasia mentioned above, other causes can explain the asymmetry such as, for example, an osteoarthritis or even a tumor emanating from the base of the skull, mandibula or soft tissues.

Hemifacial microsomia is linked to a rare homozygous variant V162I in FRK and validated in zebrafish.

OBJECTIVES: Hemifacial microsomia (HFM) is a common birth defect involving the first and second branchial arch derivatives. Although several chromosomal abnormalities and causal gene variants have been identified, genetic etiologies in a majority of cases with HFM remain unknown. This study aimed to identify genetic mutations in affected individuals with HFM. METHODS: Whole-exome sequencing and bioinformatics analysis were performed for 16 affected individuals and their family members. Sanger sequencing was applied for confirmation of selected mutations. Zebrafish embryos were used for in situ hybridization of candidate gene, microinjection with antisense morpholino, and cartilage staining. RESULTS: A homozygous missense mutation (c.484G > A; p.V162I) in the FRK gene was identified in an 18-year-old girl with HFM and dental abnormalities. Heterozygous mutation of this mutation was identified in her parents, who are first cousins in a consanguineous family. FRK is highly expressed in the Meckel's cartilage during embryonic development in mouse and zebrafish. Knockdown of frk in zebrafish showed a lower length and width ratio of Meckel's cartilage, abnormal mandibular jaw joint, and disorganized ceratobranchial cartilage and bone. CONCLUSIONS: We identified a recessive variant in the FRK gene as a novel candidate gene for a patient with HFM and mandibular hypoplasia and revealed its effects on craniofacial and embryonic development in zebrafish.

Congenital mandibular coronoid process hyperplasia and associated diseases.

Coronoid process hyperplasia (CPH) is an oral and maxillofacial surgical disease that can result in restricted jaw movement due to an enlarged and elongated mandibular coronoid process. It is characterized by the painless progressive restriction of unilaterally or bilaterally mouth opening. Clinically, unexplained bilateral CPH is less common and therefore often overlooked or misdiagnosed, and coronoidectomy can be very effective on improving mouth opening. Currently, the exact etiology and mechanism of congenital CPH have not yet been fully understood, but it is generally believed to be genetically related. In this paper, the relationship of the congenital mandibular CPH with the related diseases was examined based on cases collected in our clinic and literature review for the clinical diagnosis and treatment of patients with restricted mouth opening associated with CPH.

Morphological integration in inferior alveolar canal and mandibular shapes.

OBJECTIVE: During embryogenesis of mandible, the initial ossification centre begins at the bifurcation of the inferior alveolar (IA) and the mental nerves. Additionally, in congenital anomalies like craniofacial microsomia (CFM), the IA canal is completely absent on the microsomic side. These observations led us to hypothesise that there may be a morphological integration between these structures - the IA nerve and the mandibular shapes. Therefore, the primary objective of this study was to test for morphological integration between these structures and the secondary objective was to determine if there were shape variations in these structures among skeletal Classes I, II and III subjects. SETTING AND SAMPLE POPULATION: The sample size of the study is 80 full-head cone-beam computed tomography (CBCT) scans (age 16-56 years). METHODS: We retrieved CBCT scans from our archived database using specific inclusion/exclusion criteria. In the de-identified CBCT scans, traditional coordinate landmarks and sliding semi-landmarks were placed on the mandible and the IA canal (proxy for IA nerve). Using geometric morphometric analyses, we tested integration between the IA canal and the mandibular shapes. We used Procrustes ANOVA to test for overall shape variations among the three skeletal classes (Classes I, II and III). RESULTS: The IA canal and posterior/inferior border of mandible showed strong integration (r-PLS = .845, P = .001). Similar strong integration was also observed between the IA canal and the overall shape of the mandible (r-PLS = .866, P = .001). Additionally, there was a statistically significant variation in overall shape between skeletal Class I and Class II (P = .008) and Class II and Class III (P = .001). CONCLUSIONS: The strong integration between two structures suggests that the IA nerve may play a role in establishing mandibular shape early in development. We posit this may be important in driving mandibular defects seen in CFM, which warrants further investigation.

Is a Difficult Airway Team Needed for Intubation at Removal of Mandibular Distraction Devices for Infants With Robin Sequence?

PURPOSE: Difficult airway teams (DATs) are typically present to assist intubation at the initial mandibular distraction osteogenesis (MDO) operation for infants with Robin sequence (RS). In some institutions, the RS diagnosis triggers a "difficult airway" label for the infant, requiring DAT presence for future operations. By the time of distractor removal, however, breathing and airway anatomy are significantly improved. The objective of this study was to measure intubation difficulty and perioperative respiratory complications at MDO device removal as a proxy for the necessity for coordination with a DAT. METHODS: This is a retrospective study including infants with RS from 2013 to 2021 who had MDO during infancy. Patients were excluded if they had a tracheostomy or MDO device failure. Predictor variables included demographic data, comorbidities, and apnea-hypopnea indices (AHIs) from pre- and immediate post-MDO polysomnograms. The primary outcome measures were number of intubation attempts, laryngoscopy grade, and perioperative respiratory events at the distractor removal operation. Descriptive statistics were computed including Fisher's exact, paired sample t-tests, and Wilcoxon rank tests, and P < .05 was considered statistically significant. RESULTS: The sample included 47 (60% male) patients with a mean age at MDO of 12.0 ± 15.7 weeks. Significant improvement in AHI was seen after MDO (pre-MDO: 26.8 ± 18.4 events/hour; post-MDO 2.78 ± 2.66 events/hour; P < .001). Average number of intubation attempts decreased from 2.09 ± 1.36 to 1.30 ± 0.75 (P < .001) and the most common post-MDO laryngoscopy grade was 1 (69%). There were no intraoperative and 2 (4%) minor postoperative respiratory events, both in patients with repaired congenital cardiac disease and not related to traumatic intubation. CONCLUSION: Neither difficult intubations nor perioperative respiratory events associated with intubation trauma were seen at distractor removal, suggesting that specialty airway assistance is not routinely needed after successful MDO. DAT presence should be determined on a case-by-case basis based on specific patient risk factors.

Free Fibula Flap for the Treatment of Agnathia in a 10-Year-Old With Severe Agnathia-Otocephaly Complex.

Agnathia-otocephaly complex (AOC), a first branchial arch defect, is characterized by mandibular hypoplasia or aplasia, ear abnormalities, microstomia, and macroglossia and is a rare and often fatal diagnosis. Herein, the technical considerations and details of mandibular reconstruction using virtual surgical planning (VSP) and a vascularized free fibula flap for further mandibular reconstruction in a 10-year-old boy are presented. The patient's preoperative examination was consistent with agnathia (absence of mandibular symphysis, bilateral mandibular bodies, condyles, coronoids, rami, and temporomandibular joint), severe microstomia, and a Tessier # 30 cleft (maintained to allow oral access until later in treatment). Virtual surgical planning was utilized to plan a 3-segment fibula for the reconstruction of the mandibular symphysis and bilateral body segments, and bilateral costochondral grafts were planned for the rami. To the authors' knowledge, this represents the first application of virtual surgical planning for mandibular reconstruction with a vascularized free fibula flap in a pediatric patient with severe agnathia-otocephaly complex.

Tracheostomy, the Not So Definitive Airway?: Tracheostomy Morbidity in Pediatric Craniofacial Patients.

BACKGROUND: Tracheostomy is the definitive treatment for airway management in severe cases of craniofacial-associated upper airway obstruction, like the Pierre-Robin sequence, but is associated with significant morbidity. The purpose of this study was to examine tracheostomy-associated morbidities and mortalities in craniofacial patients to identify opportunities to improve clinical care and patient prognosis. METHODS: The study was a retrospective review of pediatric craniofacial patients who were tracheostomized between 2016 and 2022. Data regarding their demographics, craniofacial diagnoses, endoscopic airway anomalies, intubation grade of view classification, tracheostomy-related complications, and causes of mortality were analyzed. RESULTS: Sixteen of the 17 tracheostomized pediatric patients had the Pierre-Robin sequence, with 5 of those patients having an additional syndromic craniofacial diagnosis. Additional airway anomalies were found in 82.4% of the patients. The mean length of hospital stay after tracheostomy was 4.08 months. Infection was the most common complication, observed in 94.1% of patients, followed by stomal granulation in 76.5% of patients. Two mortalities were observed: one following the compassionate removal of ventilator support and the other following the accidental dislodgment of the tracheostomy tube. CONCLUSIONS: Tracheostomy-related complications were observed in all craniofacial patients in this group. Compared with the general pediatric population, tracheostomized craniofacial patients may endure longer hospital stays and greater stomal granulation rates. Mandibular distraction osteogenesis may allow for tracheostomy avoidance in these patients, and future research should focus on comparing the long-term complication rates and outcomes between tracheostomy mandibular distraction osteogenesis in this challenging patient population.

Craniofacial Microsomia: New Updates in Spinal Anomalies.

INTRODUCTION: Craniofacial microsomia (CFM) involves anomalies of the first and second pharyngeal arches, mainly of the mandible, maxilla, ears, and vertebral spine. This study aimed to identify the frequency and morphology of spinal anomalies of individuals with clinical diagnoses of CFM. In addition, the correlation between spinal anomalies and craniofacial involvement was performed. MATERIALS AND METHODS: This study was a retrospective review of individuals with a clinical diagnosis of craniofacial microsomia. The inclusion criteria were a clinical diagnosis of CFM with no overlap with any other syndromes of first and second pharyngeal arches and radiograph availability in the hospital's database. Prevalence and morphology of spinal anomalies were calculated and clinical details were recorded: types of spinal anomalies and correlations according to OMENS score. RESULTS: The sample consisted of 46 individuals with a clinical diagnosis of CFM, 24 (52,2%) female and 22 (47,8%) male (1M:1F). Twenty-one (45,7%) had unilateral craniofacial involvement and 25 (54,3%), bilateral. Twenty-eight (60,9%) individuals presented spinal anomalies. Those with unaltered spinal morphology showed a slight preference toward OMENS scores under 5: 7 patients did, only one of which had spinal alterations (14,3%); 68,8% (22) in the group with scores 5 to 9 (n=32) and 71,4% (5) in the 10 to 15 group (n=7) did as well. DISCUSSION AND CONCLUSIONS: Spinal anomalies in individuals with CFM are more common than usually reported in medical literature, mainly when associated with radial anomalies and correlate with statistical significance to facial features, mainly the OMENS score.

Himi-Lengthening Genioplasty: A Modified Technique for the Correction of Mandibular Asymmetry.

Mandibular asymmetry is among the most common facial anomalies. Traditionally, mandibular asymmetry with malocclusion has been treated with orthognathic surgery and genioplasty. However, routine genioplasty cannot achieve a satisfactory contour. Hence, this study presents a modified technique, himi-lengthening genioplasty, to resolve this matter. By combining this technique with orthognathic surgery, the authors successfully corrected mandibular asymmetry in 1-stage surgery, achieved ideal occlusion, and reconstructed the esthetic contour. No complications occurred during the 6-month follow-up period. Therefore, the authors recommend our modified surgical technique for its effectiveness, security, stability, and simplicity.

Feeding Outcomes After Mandibular Distraction for Airway Obstruction in Infants.

Robin sequence is a congenital issue resulting in airway obstruction, difficulty feeding, and failure to thrive. Mandibular Distraction Osteogenesis is used to improve airway obstruction in these patients, but little data exists characterizing feeding outcomes following surgery. This study aims to evaluate feeding outcomes and weight gain following mandibular distraction for airway correction in infants. A single-center retrospective chart review was conducted, and patients under 12 months old who underwent mandibular distraction between December 2015 and July 2021 were included in the study. The presence of cleft palate, distance of distraction, and polysomnography results were recorded. The primary outcomes were the length of distraction, need for nasogastric tube or G-tube at discharge, time lapsed to achieve full oral feeds, and weight gain (kilogram). Ten patients met the criteria. Of those 10 patients, 4 were syndromic, 7 had a cleft palate, and 4 had a congenital cardiac diagnosis. The average length of stay postsurgery was 28 days. Eight patients achieved full oral feeds in an average of 65.6 days. Five patients required nasogastric tube or G-tube at discharge, with 3 of these patients later transitioning to full oral feeds. All patients gained weight 3 months postsurgery with an average of 0.521 kg/mo. Patients who achieved full oral feeds gained an average of 0.549 kg/mo. Patients with supplementation gained an average of 0.454 kg/mo. All patients demonstrated improvement in airway obstruction with an average postoperative apnea hypopnea index of 1.64. Further investigation is necessary to identify challenges seen in feeding after mandibular distraction osteogenesis and improve care.

Application of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review.

Congenital fusion of the jaws (syngnathia) is a rare facial malformation with an unknown etiology. This disease may vary in severity with adhesion of soft tissue and bony fusion. It can be anterior fusion, unilateral or bilateral fusion, and complete fusion. The main problem of these patients is the difficulty of airway maintenance and feeding, and the most common postoperative complication is the relapse of bony fusion. Here, we report a young male patient with bony syngnathia, involving bilateral fusion of the ascending ramus and body of the mandible with the maxillary complex. We performed bone isolation by computer-assisted preoperative planning and used an insertional temporalis flap to fix the wound surface to prevent refusion of bone.

[Lower jawbone structures symmetry evaluation using cone beam computed tomography]. / Otsenka simmetrichnosti kostnykh struktur nizhnei chelyusti po dannym konusno-luchevoi komp'yuternoi tomografii.

OBJECTIVE: To assess the prevalence and quantitative characteristics of skeletal asymmetries of the body and lower jaw branches in three-dimensional space. MATERIALS AND METHODS: Using depersonalized data archive, of 400 CBCT scans, forty studies were picked randomly. Patients aged 25 to 35 years participated in research, no history of maxillofacial reconstructive surgery; gender, race, and facial anomaly were ignored. Three experienced doctors, independently, identified 15 reference points on radiographic images of AUTOPLAN software. Expert consensus found in the orientation determination. Six planes and eight points were constructed, using Math algorithms and the Python OOP; body and branches of the lower jaw length measurements performed and processed automatically. RESULTS: The study identified symmetry, using a method for determining parameters of the lower jaw in 3D space (Priority Reference No.2023100466 at 10.01.2023), modern dentistry research method. Both sides of data obtained shown asymmetry of the body and branches of the lower jaw occurred in 45% (18 people) and 67.5% (27 people) of cases. Forty percent of cases were noted with Mild asymmetry of the lower jaw body (16 people), moderate asymmetry noted in 5% (2 people). These parameters were 57.5% for branches, (23 people) and 10% (4 people). Combined form of anomaly observed in 37.5% (15 people) of cases, among which 12 people (75%) showed adaptive compensation of anomalies, which requires further investigation on a larger number of patients. CONCLUSION: Lower body jaw asymmetry is prevalent (80%). Patients with moderate and serious anomaly form appeared in 15% of cases.